Churg-Strauss vasculitis

Churg-Strauss vasculitis (CSS)/allergic (eosinophilic) granulomatosis vasculitis:
• systemic necrotising vasculitis in those with pre-existing allergic rhinitis, sinusitis or asthma – affects small and medium sized arteries of, most often, the skin, peripheral nerves and lungs.
• M>F. Onset ages 20 to 40.
• main pathologic features are necrotising vasculitis, tissue infiltration with eosinophilia and extravascular granuloma formation
• present with worsening respiratory symptoms
• course is variable; occurs after two long prodromic phases  several years for diagnosis
• American College of Rheumatology criteria for diagnosis is when 4 of 6 symptoms are present (asthma, eosinophilia, neuropathy, pulmonary infiltrates, paranasal sinus abnormality, extravascular eosinophils)
• joint symptoms in 50% – polyarticular and migratory – joints swollen and effusions; often occur early in course
• Peripheral neuropathies are common
• BEAN SAP – Blood Eosinophilia, Asthma, Neuropathy, Sinus abnormalities, Allergies, Perivascular eosinophils
• Treatment – corticosteroids and cytotoxic agents.